Poster 1012: Severe Primary Pulmonary Hypertension Complicated by Tricuspid Insufficiency, Uterine Myomatosis, and Multisystem Failure: Evolution and Management Context of Mixed Shock

Background: Primary pulmonary hypertension a rare, severe clinical condition characterized by progressive increase in pulmonary arterial pressure, which affects the vasculature and can result in right heart failure and significant hemodynamic disturbances. Early diagnosis and appropriate management are crucial to improving the quality of life and survival of patients.

Methods: To illustrate the complexity in managing severe pulmonary hypertension in the context of comorbidities, as well as the medical and surgical challenges that arise when treating patients with multiple conditions that develop multisystem involvement.

Outcome: A 40-year-old female with a history of primary pulmonary hypertension (PPH) since 2008, treated with Sildenafil, Macitentan, and home oxygen therapy, and with a surgical history including cesarean section at 32 weeks of gestation and percutaneous closure of an atrial septal defect (ASD) with an Amplatzer device in 2019. Over the years, she has presented complications such as severe tricuspid insufficiency due to chordal rupture, deterioration in functional class, and progressive dyspnea. In August 2024, the patient developed a picture of heavy vaginal bleeding and renal pain, being diagnosed with uterine myomatosis, hematometra vs pyometra, and mixed shock (hypovolemic and septic). After being admitted to the ICU and receiving intensive treatment, she underwent subtotal hysterectomy, but her condition worsened with multiple organ failure, renal and cardiovascular insufficiency. Despite resuscitation efforts, the patient experienced a cardiopulmonary arrest and died on September 1, 2024, due to irreversible multisystem and cardiovascular failure.

Conclusion: We present a patient with severe primary pulmonary hypertension and tricuspid insufficiency who developed a critical condition due to recurrent surgical complications, a septic crisis from uterine myomatosis, and persistent bleeding. Despite medical and surgical management, she died from cardiopulmonary arrest in the context of mixed shock and multisystem failure.